Desmoplastic Small Round Cell Tumor of the Kidney in Childhood

Wang, Larry L.; Perlman, Elizabeth J.; Vujanić, Gordan; Zuppan, Craig W.; Bründler, Marie Anne; Cheung, Christine; Calicchio, Monica L.; DuBois, Steven G.; Cendron, Marc; Murata-Collins, Joyce; Wenger, Gail D.; Strzelecki, Donna; Barr, Frederic G.; Collins, Tucker; Perez‐Atayde, Antonio R.; Kozakewich, Harry

doi:10.1097/01.pas.0000213432.14740.14

Cited by 74 publications

(57 citation statements)

References 25 publications

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“…DSRCT is specifically associated with t(11;22)(p13;q12), making this rearrangement a diagnostic marker of the disease [10]. Qualitative RT-PCR analysis of our tumor sample demonstrated the absence of this transcript, as well as EWS/ERG and EWS/ FLI1, which have been described in some hybrid forms that resemble DSRCT [11] and PNET, respectively.…”

Section: Discussionmentioning

confidence: 92%

“…Total RNA was extracted with Trizol Reagent 1 and reverse transcribed to cDNA using the High-Capacity cDNA Archive kit (Applied Biosystems, Foster City, CA, USA). Qualitative RT-PCR for the detection of EWS-WT1, EWS-FLI1, and EWS-ERG fusion transcripts was done according to Wang et al [10]. Results were negative for all rearrangements (Fig.…”

Section: Pathologists From Both Institutions Lastly Agreed With the Dmentioning

confidence: 99%

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Pitfalls in the differential diagnosis of renal tumor in an adolescent

Valera

Brassesco

Cortez

et al. 2009

Pediatric Blood & Cancer

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The differential diagnosis of renal tumors, particularly in adolescents, may be challenging. We describe an 11‐year‐old female with a primary intra‐renal mass. Initial differential diagnoses included primitive neuroectodermal tumor (PNET), desmoplastic small round cell tumor (DSRCT), and Wilms Tumor (WT). Extensive pathologic and molecular analysis on initial and relapsed tumor samples confirmed WT. The EWS‐WT1 and EWS‐FLI1 rearrangements, distinctive of DSRCT and PNET were negative. The differential diagnosis on monophasic blastemal WT may be complex. Primary renal DSRCT and PNET have been rarely described. Nevertheless, molecular confirmation for these rare conditions may be necessary in selected cases. Pediatr Blood Cancer 2010;54:319–321. © 2009 Wiley‐Liss, Inc.

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Section: Discussionmentioning

confidence: 92%

Section: Pathologists From Both Institutions Lastly Agreed With the Dmentioning

confidence: 99%

Pitfalls in the differential diagnosis of renal tumor in an adolescent

Valera

Brassesco

Cortez

et al. 2009

Pediatric Blood & Cancer

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“…Originally, described as a predominantly intra-abdominal tumor, a broader distribution has emerged gradually that includes pleura [61] and tunica vaginalis [62]. Extraserous location has been occasionally reported, including parotid gland [63], posterior cranial fossa [64], central nervous system [65], bone and soft tissue [66][67][68], ovary [69], pancreas [70], and kidney [71,72].…”

Section: Desmoplastic Small Round Cell Tumormentioning

confidence: 97%

Soft tissue tumors associated with EWSR1 translocation

2009

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The Ewing sarcoma breakpoint region 1 (EWSR1; also known as EWS) represents one of the most commonly involved genes in sarcoma translocations. In fact, it is involved in a broad variety of mesenchymal lesions which includes Ewing's sarcoma/peripheral neuroectodermal tumor, desmoplastic small round cell tumor,clear cell sarcoma, angiomatoid fibrous histiocytoma, extraskeletal myxoid chondrosarcoma, and a subset of myxoid liposarcoma. The fusion products between EWSR1 and partners usually results in fusion of the N-terminal transcription-activating domain of EWSR1 and the C-terminal DNA-binding domain of the fusion partner, eventually generating novel transcription factors. EWSR1 rearrangement can be visualized by the means of fluorescence in situ hybridization (FISH). As soft tissue sarcomas represent a diagnostically challenging group, FISH analysis is an extremely useful confirmatory diagnostic tool. However, as in most instances a split-apart approach is used, the results of molecular genetics must be evaluated in context with morphology.

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“…7 DSRCT is aggressive, malignant, typically affects children 6 to 8 years of age but may also be seen in young adolescents, 18 and is diagnosed by its characteristic EWS-WT1 translocation. 116 CT may reveal a hypovascular, heterogeneous, and well-circumscribed mass with internal punctate calcifications. 117 It reveals similar histologic features as in the extrarenal sites consisting of nests, cords, or sheets of small undifferentiated cells with foci of necrosis, calcification, and desmoplasia.…”

Section: Anaplastic Sarcomamentioning

confidence: 99%

An Approach to Renal Masses in Pediatrics

Malkan¹,

Loh²,

Bahrami³

et al. 2015

Pediatrics

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Renal masses in children may be discovered during routine clinical examination or incidentally during the course of diagnostic or therapeutic procedures for other causes. Renal cancers are rare in the pediatric population and include a spectrum of pathologies that may challenge the clinician in choosing the optimal treatment. Correct identification of the lesion may be difficult, and the appropriate surgical procedure is paramount for lesions suspected to be malignant. The purpose of this article is to provide a comprehensive overview regarding the spectrum of renal tumors in the pediatric population, both benign and malignant, and their surgical management.

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Desmoplastic Small Round Cell Tumor of the Kidney in Childhood

Cited by 74 publications

References 25 publications

Pitfalls in the differential diagnosis of renal tumor in an adolescent

Pitfalls in the differential diagnosis of renal tumor in an adolescent

Soft tissue tumors associated with EWSR1 translocation

An Approach to Renal Masses in Pediatrics

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